Chapter 5
Living with Hemophilia—An Accident Waiting to Happen

Despite the normality of life for most hemophiliacs in the twenty-first century, hemophiliacs still face many challenges and decisions throughout their lives. Decisions can range from a child deciding on a sport to participate in, to a young adult with a family history of hemophilia considering its impact on a decision to have children. The challenge of hemophilia is one for the entire family, not just the patient, since all family members are affected by living with a person with a chronic illness. However, wise choices, made from sound information, give the best chance for a normal life for the patient as well as the family.

Sports

One of the decisions that hemophiliacs face is what sports are safe, given the severity of their hemophilia. Those with mild hemophilia have more choices than those with severe hemophilia. The majority of hemophiliacs are aware that participation in sports is beneficial as long as sensible choices are made, because regular exercise keeps joints strong by providing muscular support. Swimming is the number one sport universally agreed by physicians as safe for hemophiliacs. Swimming protects joints by developing muscles around them, but does not put undue strain on them. The risk of being bumped or jostled is low compared with other sports. Golf is also a sport most physicians agree is safe for even severe hemophiliacs. Perry Parker is a hemophiliac who plays golf professionally. He also works with young boys with hemophilia to teach them how to safely participate in sports by demonstrating proper techniques. Perry has a brother, Corey, also a hemophiliac, who is a professional baseball player.

According to the Canadian Hemophilia Society, other sports that put low stress on joints are bicycling, tennis, and running. Paul McNeil is a registered nurse in Canada who has hemophilia B (Christmas disease). With careful training, he was able to run the Canadian International Marathon in 2000, but agrees that the stress of a marathon would not be suitable for all hemophiliacs. Weight lifting, using light to medium handheld weights, can be helpful in building muscles as long as proper technique is employed. Weight lifting with heavy weights is not recommended because of the danger of injuring muscles that provide the support for joints.

Those with mild hemophilia may be able to play soccer or baseball, or even ski. Skateboarding, rollerblading, and horseback riding, however, are all considered high-risk sports because the risk of falling with resultant serious injury is higher than for other activities. Hemophiliacs are warned not to participate in sports when their joints are swollen and painful.

Certain physical activities are considered off-limits for all with hemophilia. These activities are mostly contact sports like football, wrestling, boxing, and ice hockey. Participation in contact sports puts a high degree of stress on joints and can lead to bleeding episodes.

Depending on the sport, protective gear may be needed for athletic hemophiliacs. Fortunately, today most children wear helmets, pads, or gloves when biking or batting at baseball, so this is not considered unusual. It is not necessary to wear protective gear for activities that normally do not require it. Many medical professionals believe that this practice puts too much focus on the illness and is counterproductive.

Developmental Issues

Another area that impacts children with hemophilia is developmental milestones and how those affect a child with hemophilia. Each new age level brings different issues regarding the illness.

How children cope with hemophilia is largely determined by age. Preschoolers, who have limited understanding, often cry or fight back during treatments requiring a needle. To cope with such treatment, which preschoolers sometimes see as a form of punishment, they often revert to comforting behavior like thumb sucking.

Full understanding of why treatment is necessary usually does not happen until age six or seven. School-aged children are old enough and knowledgeable enough to recognize and report signs of internal bleeding.

Adolescence is a difficult time for a hemophiliac because teenagers want to fit in, not stand out as different. Some teen hemophiliacs deliberately engage in risky behavior in an attempt to deny the seriousness of their disorder. Audrey Taylor, a hemophilia nurse coordinator, says, "One of the biggest concerns with this age group is letting bleeds get out of hand because they don't want to rest or miss activities."³⁴ In addition, emotional feelings concerning hemophilia can peak during this age.

The limitations put on lifestyle by hemophilia can damage self-esteem, which is the amount of worth people attribute to themselves. Children learn self-esteem early in life through success in everyday activities and through treatment by other people. Hemophiliacs recognize early that their life is different from others and may feel afraid, powerless, or angry. Having strong support from family members is important in coping with hemophilia. Allowing children to choose activities they excel at and enjoy helps develop good self-esteem. Failure to adapt to the disease can lead to isolation and a decrease in job and social opportunities.

School Days

Hemophilia is a disease that requires information to be shared with anyone who has contact with the child for an extended time, including day care workers, school nurses, and teachers. Because of the rareness of the disease, even school nurses may have little experience with hemophilia and may need additional education.

Day care and preschool workers receive formal in-service training from an experienced hemophilia nurse. In addition, they must be knowledgeable about first aid for external bleeds. The responsibility of parents is to provide an emergency care plan in case their child has a bleed, and they must let staff know their main responsibility is to notify the parents immediately, especially when there is the possibility of an internal bleed.

Children with hemophilia can be in regular classrooms and participate in regular activities. By the time a child is school aged, he can report symptoms of internal bleeds to his teachers. The type of bleeding hemophiliacs experience always allows for time to call for help. Although the bleeding lasts longer, it is not faster, so calling 9-1-1 is usually not necessary.

Summer Camps

Camps for hemophiliacs are designed to provide a sense of normalcy by planning outdoor fun and a chance to meet other hemophiliac children and their families. Nearly every state has one camp available for hemophiliacs. The National Hemophilia Foundation provides a list of available camps with information about each one.

Most camps last a week, are staffed with professional medical people, and admit campers ages six to nineteen, depending on the camp. Some camps also sponsor educational programs for parents while their children enjoy activities such as talent shows, boating, swimming, hiking, fishing, and crafts. Many camps teach willing children to self-infuse clotting factors for the first time as part of the week's activities.

Costs for a week of camp can range from free to hundreds of dollars. However, many camps receive supplemental funds from state hemophilia organizations, corporations, and individuals and are able to offer very low-cost camping. Actor Paul Newman contributes money for camps for children with serious illnesses. These camps, named Hole in the Wall Gang, are located in Connecticut and Florida.

Careers

One of the big steps in becoming independent is choosing a career. Hemophiliacs have nearly as many job opportunities as others, with only a few exceptions. Those with hemophilia generally are not good candidates for hazardous jobs where injury is a possibility. However, manual labor, for the most part, does not have to be avoided.

According to the National Hemophilia Foundation, those with severe hemophilia have sometimes encountered discrimination by prospective employers, although it is illegal to discriminate because of physical problems as long as a person is capable of performing the job. Val Bias of California says, "I only told my immediate supervisor about my hemophilia after I had the job and usually after I had proven myself in some way. If it wasn't hazardous work, or if my hemophilia wouldn't interfere with my ability to do the work, then I didn't tell. People may disagree with this philosophy, but my career is not about hemophilia, it is about my ability, my competence, and my skill."³⁵

Marriage and Having Children

Along with choosing a career, marriage and a family are important. A person with a family history of hemophilia might wish to consult a genetic counselor before having children. A genetic counselor is a medical professional with special training in diseases that are inherited. The counselor's job is to look at the patient's family history to determine the risk of transmitting genetic diseases to future children and then relay this knowledge to the patient. Once information is received, prospective parents can use it to decide whether they want to have children and risk transmission of the disease.

If the prospective parents choose to have children and one or more of the children has hemophilia, mothers in particular usually feel guilty, since mothers pass the gene to sons by their X chromosome. Rachel Stuart, nurse coordinator at Phoenix Children's Hospital Hemophilia Center, says, "Moms often feel responsible for their son's condition, and I tell them it's not their fault at all. You don't get to pick which genes you pass on—it's a totally random roll of the dice."³⁶

The Affect of Hemophilia on the Family

Parents of a child with hemophilia may be overprotective or permissive. Because of the potential consequences of an unchecked bleeding episode, parents often want to shield their child from any situation in which he might be injured. This type of parent sets many limits on the child's activities. An anonymous mother commented in Raising a Child with Hemophilia, "My husband became overprotective when our son began to walk. He would hover near him and expect me to hover over him, too. He tried to avoid injuries by carrying our son everywhere. But our son began to resent this. My husband became more upset than I whenever our son got hurt and felt the need to blame one of us."³⁷

The opposite situation is a parent who is too permissive. Parents who react this way attempt to make up for the restrictions of hemophilia by letting the child have or do whatever he wants. In other words, there are no rules about acceptable behavior and they are exempt from helping with routine household chores.

Hemophilia affects all family members including siblings. The brother or sister of a hemophiliac may feel neglected because of the amount of attention the affected child receives. Sisters, as they grow older and learn how the disease is transmitted, may worry about the possibility of their being carriers. Experts on hemophilia say it is common for brothers or sisters to sometimes be jealous of or be angry with the child who has hemophilia. Negative feelings are handled by allowing appropriate expression, by teaching brothers or sisters about hemophilia, and by parents setting aside time to spend with the unaffected child. Elaine Guffey of Missouri relates the effect hemophilia had on her son's older brother: "It was hard for Jimmy at first because he is the oldest, has no hemophilia, and was used to getting all the attention. I just show him he is loved and is as important to me as Brandon, in his own special way. I show that his feelings count. I talk to him at length if anything is upsetting him."³⁸

Choosing Where to Live

The decision about where to live is another issue that can cause stress for the family of a hemophiliac. Living in an area distant from a hemophilia treatment center means that a nonspecialist in hemophilia may be the only choice for care. Although there are more than 130 hemophilia treatment centers in the United States, some hemophiliacs live in rural areas, making the trip to a center time consuming. Many of these hemophiliacs use the centers only for occasional checkups and must rely on local nonspecialist physicians and emergency rooms for care. Rita Williams, the mother of a hemophiliac, related her story of living in a rural area: "The nearest hemophilia treatment center is in Loma Linda, 65 miles away. Each trip (and there are plenty) is an 8 to 10 hour ordeal. The local hospital is closer but does not carry our product, and the staff did not feel comfortable infusing my child. I finally started using homecare. This allows me to use the local hospital, at least for emergencies."³⁹

Receiving care from a physician not experienced with hemophilia can lead to errors in treatment, since the disease is rare and many doctors never treat hemophiliacs. Susan Resnik, in her book Blood Saga, quotes a man called "Cliff H." who has severe hemophilia, and was born in a small New Mexico town before hemophilia treatment centers were available. "Cliff" described his experience using a nonspecialist:

There was a family doctor who was my physician during all my growing up years—he was just a family physician, not a specialist. Certainly he did not know a lot of things that he probably should have known at the time, and frankly, probably made some errors. But by the same token, he did a lot of things right. Right in the sense that he always advised me that the person who should know the most about the disease is myself. He used to give me articles he had at the time, and his counsel was, "I don't know everything that we should be doing, but we'll do the best we can." He used an awful lot of heating pads in those days that should never have been used. And that complicated a lot of factors. But for the most part all we had was whole blood, narcotics [for pain], and bed rest.⁴⁰

Many hemophiliacs who do not live near a hemophilia treatment center keep an adequate supply of clotting factor on hand and learn home infusion.

Insurance

A final issue that must be dealt with by families of hemophiliacs is how to cover the cost of treatment. Because hemophilia is an expensive disease to treat, health insurance coverage is very important. Unfortunately, there is a downside to health insurance. In 1970, the insurance industry set a $1 million cap on lifetime coverage for an illness, meaning that is the maximum amount of money they will ever pay in a person's lifetime. Since annual treatment for an adult hemophiliac can cost between $100,000 and $200,000, it is possible to exceed the $1 million limit in five to ten years. Very few families can afford expensive hemophilia treatment without some type of financial help.

Children diagnosed with hemophilia in the first year of life will likely use their total benefits by their teen years, leaving them without insurance coverage. Children can apply for help from state-funded programs. Adults have fewer options, but may be eligible for Medicaid (a federally funded, state-operated program of medical assistance) or Social Security, which administers Medicare, a health insurance plan for people age sixty-five and over.

Hemophilia is a lifelong disease that requires the patient and family to recognize symptoms and manage treatment on an everyday basis. Patients and families with the best understanding of the disease are able to live the most normal lifestyle. Susan Cole, the mother of a child with hemophilia, has this advice. "Don't be afraid—you can handle it. Hemophilia may not be curable yet, but it's a very manageable condition."⁴¹